NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial

UniProt P49821 Organism Homo sapiens Gene NDUFV1, UQOR1 EC 7.1.1.2

Also known as: NDUFV1, UQOR1

Function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Part of the peripheral arm of the enzyme, where the electrons from NADH are accepted by flavin mononucleotide (FMN) and then passed along a chain of iron-sulfur clusters by electron tunnelling to the final acceptor ubiquinone. Contains FMN, which is the initial electron acceptor as well as one iron-sulfur cluster.

Classification

Family (Pfam): PF01512 Complex1_51K, PF10589 NADH_4Fe-4S, PF22461 SLBB_2
InterPro: ComplexI_51kDa_subunit, NADH-UbQ_OxRdtase_51kDa_CS, NADH-UbQ_OxRdtase_suF, Nuo51_FMN-bd, Nuo51_FMN-bd_sf, Nuop51_4Fe4S-bd, Nuop51_4Fe4S-bd_sf, SLBB_dom
Functional cluster: Amino-Acid & Central Metabolism Enzymes

Experimental structures · PDB · 7

5XTB EM 3.40A
5XTD EM 3.70A
5XTH EM 3.90A
5XTI EM 17.40A
9CWT EM 3.44A
9I4I EM 2.63A
9TI4 EM 2.66A

A predicted model is available from AlphaFold.

Gene Ontology · 13

Disease associations

mitochondrial complex I deficiency, nuclear type 4 MONDO:0032609

Drugs targeting this protein · 2

METFORMIN HYDROCHLORIDE inhibitor
ME-344 inhibitor

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial 1 shared papers
Cytochrome b-c1 complex subunit 2, mitochondrial 2 shared papers
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1 shared papers
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 1 shared papers
Neudesin 1 shared papers
GEL complex subunit OPTI 1 shared papers
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1 shared papers
Cytochrome b-c1 complex subunit 7 1 shared papers
Cytochrome c oxidase subunit 7C, mitochondrial 2 shared papers
Parkinson disease protein 7 homolog 1 shared papers
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1 shared papers
Cytochrome c oxidase subunit 5A, mitochondrial 2 shared papers

Literature · 14 cited papers

Rewiring of the Human Mitochondrial Interactome during Neuronal Reprogramming Reveals Regulators of the Respirasome and Neurogenesis. IScience · 2019
Architecture of human mitochondrial respiratory megacomplex I2III2IV2. Cell · 2017
N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency. Am. J. Hum. Genet. · 2001
Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning. Proc. Natl. Acad. Sci. U.S.A. · 2000
Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy. Nat. Genet. · 1999
The structure of the human NDUFV1 gene encoding the 51-kDa subunit of mitochondrial complex I. Mamm. Genome · 1999
Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation? Biochem. Biophys. Res. Commun. · 1998
… and 2 more in the literature graph

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